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    Doppler echocardiography-right heart catheterization relationships in patients with idiopathic pulmonary fibrosis and suspected pulmonary hypertension.
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    Abstract:
    Pulmonary hypertension occurs commonly in patients with interstitial lung disease but is frequently not diagnosed or, if diagnosed, not further evaluated. In this study, we wanted to determine the accuracy of right ventricular systolic pressure measured by transthoracic Doppler echocardiography compared with hemodynamic measurements taken at right heart catheterization in patients with idiopathic pulmonary fibrosis who had suspected pulmonary hypertension.Right heart catheterization was conducted if pulmonary hypertension was suggested by echocardiography-right ventricular systolic pressure >40 mm Hg. Pulmonary hypertension was defined by right heart catheterization as mean pulmonary artery pressure > or =25 mm Hg and pulmonary artery occlusion pressure < or =15 mm Hg.Right heart catheterization demonstrated pulmonary hypertension in 75% while no pulmonary hypertension was confirmed in 12%. Correlations existed between right ventricular systolic pressure with pulmonary artery systolic pressure (r=0.66; p<0.0001) and mean pulmonary artery pressure (r=0.69; p<0.0001). Bland-Altman analysis illustrated the mean difference between the two methods was 7.75 mm Hg.Pulmonary hypertension occurs commonly in IPF. Accurate classification of pulmonary hemodynamics and degree of pulmonary hypertension severity require right heart catheterization. Prospective screening Doppler echocardiography and pulmonary hypertension therapeutic interventions warrant further prospective study in idiopathic pulmonary fibrosis.
    Keywords:
    Cardiac catheterization
    Heart catheterization
    To determine the prevalence of pulmonary hypertension in patients with idiopathic pulmonary fibrosis (IPF) by noninvasive methods.Twenty seven patients with IPF attending the Chest Clinic over a period of one-and-a-half-years underwent echocardiography for evidence of pulmonary hypertension, which was defined as pulmonary artery systolic pressure > or = 40 mmHg by Doppler echocardiography, or pulmonary acceleration time < or = 100 milliseconds or two-dimensional echocardiographic findings of right ventricular hypertrophy or overload.Two patients with clinical evidence of pulmonary hypertension were excluded from analysis. Their mean age (n = 25) was 53.8 years. The mean duration of symptoms before presentation was 2.1 years. Pulmonary hypertension was diagnosed by echocardiography in nine of the 25 patients (36%). There was a statistically significant difference between the duration of illness, arterial oxygen tension (PaO2) levels and forced vital capacity in patients with pulmonary hypertension than in those without pulmonary hypertension. Logistic regression analysis showed a significant association between the duration of illness, decreasing forced vital capacity and hypoxemia with the development of pulmonary hypertension.Echocardiography detects pulmonary hypertension in clinically asymptomatic individuals, and should be used routinely for the diagnosis of pulmonary hypertension in patients with IPF.
    Right ventricular hypertrophy
    Citations (23)
    Idiopathic pulmonary fibrosis (IPF) is a disease with very high mortality.We sought to characterize serial changes in pulmonary artery pressures (PAP) in patients with advanced IPF who survive to transplant.Retrospective analysis of IPF patients comparing mean PAP at the time of initial evaluation for transplan- tation (mPAP(baseline)) with mPAP at the time of transplant (mPAP(follow-up)). The measurements were correlated with New York Heart Association (NYHA) functional class and oxygen requirements.The final cohort consisted of 44 patients with serial right heart catheterization data. The mean mPAP(baseline) and mPAP(follow-up) were 22.5 and 32.7 mm Hg, respectively. 38.6% (17/44) of the patients had pulmonary hypertension (PH) at baseline. The majority of the non-PH patients developed PH during the serial time interval with a subsequent incidence of 77.8%. At the time of transplant, 86.4% of the patients had PH. There was a significant association between transplant NYHA class, severity of PH and oxygen requirements. Transplant NYHA class IV patients had a higher rate of mPAP change. The severity of PH at the time of transplant did not affect transplant outcomes.PH is common and progressive in patients with advanced IPF who are transplant candidates. Serial change and severity of PAP elevations have a significant association with oxygen requirements and functional status, but not transplant outcomes. Whether or not progressive PH has a significant impact on outcomes without transplantation requires further study.
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    In 47 patients with chronic obstructive pulmonary disease and 19 patients with idiopathic pulmonary fibrosis pulmonary haemodynamics were studied. Pulmonary arterial pressure was compared with non-invasive lung function tests, ecg and the chest X-ray. Correlations between pulmonary arterial pressure and some of those parameters were found. Investigations were repeated after 3 or 5 years depending on severity of pulmonary hypertension initially found. The progression of pulmonary hypertension was found. It was accompanied by intensification of non-invasive indices of pulmonary hypertension. More than half of patients with initially severe pulmonary hypertension (> or = 30 mmg Hg) did not survive 3 years.
    Pulmonary arterial pressure
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    Pulmonary hypertension (PH) may complicate idiopathic pulmonary fibrosis (IPF) but the prevalence of PH in IPF remains undefined. The present authors sought to describe the prevalence of PH in IPF. The lung transplant registry for the USA (January 1995 to June 2004) was analysed and IPF patients who had undergone right heart catheterisation (RHC) were identified. PH was defined as a mean pulmonary arterial pressure ( P̄ pa ) ≥25 mmHg and severe PH as a P̄ pa >40 mmHg. Independent factors associated with PH were determined. Of the 3,457 persons listed, 2,525 (73.0%) had undergone RHC. PH affected 46.1% of subjects; ∼9% had severe PH. Variables independently associated with mild-to-moderate PH were as follows: need for oxygen, pulmonary capillary wedge pressure ( P pcw ) and forced expiratory volume in one second (FEV 1 ). Independent factors related to severe PH included the following: carbon dioxide tension, age, FEV 1 , P pcw , need for oxygen and ethnicity. A sensitivity analysis in subjects with P pcw <15 mmHg did not appreciably alter the present findings. Pulmonary hypertension is common in idiopathic pulmonary fibrosis patients awaiting lung transplant, but the elevations in mean pulmonary arterial pressure are moderate. Lung volumes alone do not explain the pulmonary hypertension. Given the prevalence of pulmonary hypertension and its relationship with surrogate markers for quality of life ( e.g. activities of daily living), future trials of therapies for this may be warranted.
    Pulmonary wedge pressure
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    to estimate the comparative value of conventional radiology and cardiac catheterization in establishing the diagnosis and severity of primary and secondary pulmonary hypertension. We also tried to achieve some correlations between the radiological and invasive parameters.we performed a retrospective study over an 11-years time period, analyzing data from a group of 14 patients diagnosed with primary pulmonary hypertension compared to a matched group of 20 patients suffering from pulmonary hypertension secondary to mitral and aortic valvular disease. All the patients had undergone conventional radiology (chest X-ray) and catheterization of the right heart cavities and pulmonary artery.we detected significantly elevated pulmonary artery pressures and resistances in the primary pulmonary hypertension group compared to secondary hypertension patients. Cardiac output values were much lower in the primary pulmonary hypertension individuals compared with secondary pulmonary hypertension in left-sided valvular disease. The pulmonary artery arch diameter, the diameter of the right descending pulmonary artery and the value of the arterio-bronchial ratio were similar in the two groups.in both groups, we found a statistically significant positive correlation between the values of the pulmonary artery vascular resistance and the diameter of the right descending pulmonary artery, which are very specific parameters in the diagnosis of pulmonary hypertension. In the primary pulmonary hypertension group we identified a significant inverse correlation between the diameter of the right descending pulmonary artery and the values of cardiac output. This finding was not confirmed in the secondary group.
    Cardiac catheterization
    Right pulmonary artery
    Citations (4)
    Introduction. Idiopathic pulmonary arterial hypertension (IPAH) is rare and difficult progressive disease with prevalence of approximately 15 cases per million residents, with predominant female cases. Case Outline. A 47-year-old female presented with symptoms and signs of the right heart chambers failure. Over prior seven years the patient had the feeling of suffocation and fatigue when walking, and received treatment for bronchial asthma. Physical examination revealed a marked loud second heart sound over the pulmonary artery. Electrocardiogram: right ventricular hypertrophy. Spirometric (pulmonary capacity) test, cardiac perfusion scan and spiral CT scanning excluded secondary pulmonary arterial hypertension. Blood testing for connective tissue diseases and HIV were within normal reference limits. Transthoracic colour Doppler echocardiography demonstrated a mild tricuspid regurgitation with high values of estimated maximal and middle systolic pressure of the right ventricle (135/110 mm Hg), and excluded previous heart disease. Cardiac catheterization confirmed IPAH diagnosis, with systolic right ventricular pressure of 101/47/66 mm Hg and pulmonary capillary pressure of 30/13/10 mm Hg. Basic therapy with sildenafil, nevertheless, considerable limitations of strain tolerance was still present. Conclusion. IPAH is a severe heart disease with non-specific signs and symptoms. Screening for IPAH is transthoracic colour Doppler echocardiography shows high correlation with cardiac catheterization.
    Cardiac catheterization
    Right ventricular hypertrophy
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